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Primary undifferentiated embryonal sarcoma of the liver mistaken for hydatid disease.

Faraj W, Mukherji D, El Majzoub N, Shamseddine A, Shamseddine A, Khalife M - World J Surg Oncol (2010)

Bottom Line: Primary undifferentiated embryonal sarcoma of the liver is a rare tumor with a peak incidence between the ages of 6 and 10 years.We report a case of a primary hepatic undifferentiated embryonal sarcoma arising in a 21-year-old male mistaken for hydatid disease of the liver.The rapid recurrence of this tumor along the site of attempted percutaneous drainage illustrates some important management points regarding this malignancy.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Surgery, HPB and liver transplantation unit, American University of Beirut Medical Center, Beirut, Lebanon. wfaraj@hotmail.com

ABSTRACT
Primary undifferentiated embryonal sarcoma of the liver is a rare tumor with a peak incidence between the ages of 6 and 10 years. We report a case of a primary hepatic undifferentiated embryonal sarcoma arising in a 21-year-old male mistaken for hydatid disease of the liver. The rapid recurrence of this tumor along the site of attempted percutaneous drainage illustrates some important management points regarding this malignancy.

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Intra-abdominal lesion extending through the drain site 5 cm outside the skin.
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Figure 3: Intra-abdominal lesion extending through the drain site 5 cm outside the skin.

Mentions: A technically challenging extended right hepatectomy was performed. Histopathology revealed an unusual neoplasm composed of irregular spindle cells showing moderate pleomorphism and brisk mitotic activity (Figure 2). Immunohistochemical studies showed positive staining for vimentin and a positive cytokeratin AE1/3. Diagnosis was made of a stage III undifferentiated embryonic sarcoma (UES). The patient was discharged home day 16 post-resection. Adjuvant chemotherapy was recommended and the patient elected to be treated in his native country. Three weeks post-discharge he re-presented to our institution with a fungating abdominal wall mass at the site of previous percutaneous drain insertion. CT scan revealed a new lesion in segment II and an intra-abdominal lesion extending through the drain site 5 cm outside the skin (Figure 3). The skin lesion was resected and histopathological examination revealed metastatic UES. He elected to go back to his country of origin where he received adjuvant chemotherapy which was ifosfamide plus etoposide alternating with actinomycin-D, vincristine every 3 weeks for 6 months. He is currently 5 months post-resection and a recent CT scan showed no evidence of disease recurrence.


Primary undifferentiated embryonal sarcoma of the liver mistaken for hydatid disease.

Faraj W, Mukherji D, El Majzoub N, Shamseddine A, Shamseddine A, Khalife M - World J Surg Oncol (2010)

Intra-abdominal lesion extending through the drain site 5 cm outside the skin.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2913916&req=5

Figure 3: Intra-abdominal lesion extending through the drain site 5 cm outside the skin.
Mentions: A technically challenging extended right hepatectomy was performed. Histopathology revealed an unusual neoplasm composed of irregular spindle cells showing moderate pleomorphism and brisk mitotic activity (Figure 2). Immunohistochemical studies showed positive staining for vimentin and a positive cytokeratin AE1/3. Diagnosis was made of a stage III undifferentiated embryonic sarcoma (UES). The patient was discharged home day 16 post-resection. Adjuvant chemotherapy was recommended and the patient elected to be treated in his native country. Three weeks post-discharge he re-presented to our institution with a fungating abdominal wall mass at the site of previous percutaneous drain insertion. CT scan revealed a new lesion in segment II and an intra-abdominal lesion extending through the drain site 5 cm outside the skin (Figure 3). The skin lesion was resected and histopathological examination revealed metastatic UES. He elected to go back to his country of origin where he received adjuvant chemotherapy which was ifosfamide plus etoposide alternating with actinomycin-D, vincristine every 3 weeks for 6 months. He is currently 5 months post-resection and a recent CT scan showed no evidence of disease recurrence.

Bottom Line: Primary undifferentiated embryonal sarcoma of the liver is a rare tumor with a peak incidence between the ages of 6 and 10 years.We report a case of a primary hepatic undifferentiated embryonal sarcoma arising in a 21-year-old male mistaken for hydatid disease of the liver.The rapid recurrence of this tumor along the site of attempted percutaneous drainage illustrates some important management points regarding this malignancy.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Surgery, HPB and liver transplantation unit, American University of Beirut Medical Center, Beirut, Lebanon. wfaraj@hotmail.com

ABSTRACT
Primary undifferentiated embryonal sarcoma of the liver is a rare tumor with a peak incidence between the ages of 6 and 10 years. We report a case of a primary hepatic undifferentiated embryonal sarcoma arising in a 21-year-old male mistaken for hydatid disease of the liver. The rapid recurrence of this tumor along the site of attempted percutaneous drainage illustrates some important management points regarding this malignancy.

Show MeSH
Related in: MedlinePlus